Attention difficulties and physical dysfunction common in children with complex congenital malformations: a study of preschool children with VACTERL association (original) (raw)
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Developmental Medicine & Child Neurology, 2010
MDI Mental Developmental Index PDI Psychomotor Developmental Index SES Socioeconomic status TIS Total impairment score AIM The aim of this study was to evaluate cognitive and motor development in children with major congenital anomalies and the predictability of development at age 5 years. METHOD A prospective, longitudinal follow-up study was undertaken. The Dutch version of the Bayley Scales of Infant Development -Mental Developmental Index (MDI) and Psychomotor Developmental Index (PDI) -were administered at the ages of 6, 12, and 24 months. The Revised Amsterdam Children's Intelligence Test -IQ and the Movement Assessment Battery for Children -Total impairment score (TIS) were used at age 5 years. A total of 117 children participated in the study. After excluding 12 children who had a major chromosomal or syndromal abnormality, the analysis was limited to 105 children (50 females, 55 males). Seven groups of congenital anomalies were distinguished: (1) small intestinal anomalies; (2) abdominal wall defects, comprising gastroschisis and omphalocele; (3) oesophageal atresia; (4) congenital diaphragmatic hernia; (5) Hirschsprung disease; (6) anorectal malformations; and (7) miscellaneous diagnoses. Logistic regression analyses served to determine the ability of MDI and PDI to predict IQ and TIS at age 5 years.
Journal of Pediatric Surgery, 2013
Background/purpose: To evaluate growth and development in children with anorectal malformations and to analyze effects of type of malformation and comorbidities. Methods: Non-syndromal children with anorectal malformations were prospectively evaluated at 0.5, 1, 2, and 5 years. Biometrics were obtained at all visits. Mental and psychomotor function development was determined. Results: 108 children (59% male) were included. 49% had a high malformation, and 46% had ≥ 1 additional major comorbidity. All growth parameters were below the norm at all ages (p b 0.01), irrespective of type of malformation. Children with ≥ 1 additional major anomaly had lower height at all ages; at 5 years, mean (95% CI) height was − 1.83 (− 2.7 to − 1.1) and − 0.70 (− 1.3 to − 0.1) in children with and without comorbidities, respectively (p = 0.019). Mental development was normal, irrespective of the type of malformation or comorbidities. Motor development was delayed at all ages. At 5 years, motor development (n = 30) was normal in 70%, borderline in 23%, and 7% had definitive motor problems (p = 0.043). Conclusion: Non-syndromal children with anorectal malformations are at risk for growth impairment, especially those with additional major comorbidity. Mental development is normal. Motor development is slightly impaired. Supportive care should focus on growth, dietary management, and motor development besides defecation problems.
Neurodevelopmental outcomes in individuals with VACTERL association. A population-based cohort study
PLOS ONE, 2023
Background Studies on neurodevelopmental outcomes in individuals with congenital anomalies who undergo neonatal surgery are scarce and have reported contradictory findings based on small study groups. The congenital condition VACTERL association includes at least three malformations: vertebral anomalies, anorectal malformations, cardiac defects, tracheoesophageal fistula with or without esophageal atresia, renal anomalies and limb deformities. Most of these patients undergo surgery during their first days of life. Neurodevelopmental disorders include a broad group of disabilities involving some form of disruption to brain development. Attention deficit hyperactivity disorder (ADHD), autism spectrum disorders (ASD) and intellectual disability (ID) are diagnoses included in this group. The aim of the study was to investigate the risk of ADHD, ASD and ID in a cohort of individuals with VAC-TERL association. Method Data was obtained from four Swedish national health registers and analyzed using the Cox proportional hazards model. Patients born 1973-2018 in Sweden with the diagnosis of VAC-TERL association were included in the study. For each case five healthy controls matched for sex, gestational age at birth, birth year and birth county were obtained. Results The study included 136 individuals with VACTERL association and 680 controls. Individuals with VACTERL had significantly higher risk of ADHD, ASD and ID than the controls; 2.25 (95% CI, 1.03-4.91), 5.15 (95% CI, 1.93-13.72) and 8.13 (95% CI, 2.66-24.87) times respectively.
2011
LIST OF PAPERS V 1. BACKGROUND II Øivind Solberg and Maria Teresa Grønning Dale, thank you both for all the fun! The "heartkids" study was truly enriched when you joined us with your project on the "heartmoms". Thank you for the daily laughs, conversations, debriefings and discussions. Bo Engdahl, you joined the heart group in the last year of my PhD. Thank you for contributing with your statistical expertise and your warm company. Over the last three years my workplace has been the Division of mental health. I would like to thank Director Arne Holte for providing a motivating, inspiring and enjoyable environment. Anne Karine, Kari, Cathrine and Grethe deserve special thanks for making everyday life at the division much easier. It is also such a privilege to be a part of an enthusiastic group of PhD students, Post docs and researchers all contributing with their knowledge and helpful advice here at the division. I am especially grateful to my coworkers at the Department of Psychosomatics and Health Behaviour for being so supportive and inclusive. Eivind, a warm thank you for all the time you have spent discussing methodological issues with me, and especially for helping out with the IRT analysis. Mari and Helga, thank you for being the best colleagues and friends anyone could have, and for always knowing when I needed-a good long coffee break.
Assessment of post-surgical functional outcome in children with anorectal malformation
International Surgery Journal, 2018
Background: Anorectal malformations (ARMs) comprise a spectrum of congenital anomalies that continue to present a challenge for paediatric surgeons. Advances in modern surgical techniques and neonatal care have greatly improved survival among ARM patients over the last decades, and early mortality is now unusual in the absence of fatal associated cardiac or chromosomal defects. The aim of this study is to measure the functional outcome of ARM by most recent krickenburg classification.Methods: The present longitudinal study was conducted in Department of paediatric surgery, Dr. BRAM hospital, Raipur, Chhattisgarh during study period February 2016 to September 2017. Those patients who had completed their all stages of surgery for anorectal malformation at-least 6 months back and arriving at outpatient department of paediatric surgery were included.Results: Maximum number of patients were in age group of 3 to 5 years (77.8%) and minimum were 9 to 11 years (3.7%). A 50.6% male and 49.4%...
International Journal of Adolescent Medicine and Health, 2020
Objectives Transitional care for adolescents with congenital malformations, such as anorectal malformations (ARM), is described sparsely in the literature and referred to as being inadequate. In order to organize future successful healthcare structures, knowledge of patient-reported important aspects of transition is required. The aim of the study was therefore to explore the needs and expectations of transitional- and adult healthcare among adolescents and adults born with ARM. Methods Two tertiary paediatric surgical centres, in collaboration with two tertiary pelvic floor centres, in Sweden and Norway, conducted a qualitative study, involving adolescents and adults born with ARM in focus group discussions regarding transitional care. Discussions were analyzed by qualitative content analysis. Ethical approval was obtained. Results Sixteen participants (10 women) with a median age of 24 (19–47) years, born with mixed subtypes of ARM were included in gender-divided focus groups. Par...
Spectrum of Congenital Anomalies among Surgical Patients at a Tertiary Care Centre over 4 Years
International Journal of Pediatrics, 2017
Introduction. Congenital anomalies are important causes of childhood death, chronic illness, and disability in many countries. Congenital malformations are rapidly emerging as one of the major worldwide problems. Aim. To study the percentage of various congenital anomalies among the patients admitted in Department of Pediatric Surgery at a tertiary care centre over a period of four years from 2011 to 2015 in our centre. Results. Neural tube defects were found to be the most common anomalies in 24.3% of the children admitted. Other common anomalies were anorectal malformation (20.7%), tracheoesophageal fistula (20%), and intestinal obstruction (14.84%). Majority (60.5%) of the patients were males. Conclusion. Congenital malformations are rapidly emerging as one of the major worldwide problems as they can result in long-term disability, which may have significant impacts on individuals, families, health-care systems, and societies. Regular antenatal visits and prenatal diagnosis are r...
OBJECTIVES. The purpose of this work was to examine changes in quality of life, disease-specific functioning, and psychosocial competencies of children and adolescents (8 -16 years of age) with anorectal malformations or Hirschsprung disease and to identify predictors of change in quality of life by testing an explanatory model in which background variables explained changes in quality of life via changes in disease-specific functioning and psychosocial competencies.
Congenital anomalies: hospitalization in a pediatric unit
OBJECTIVE:To analyze the profile of children with congenital anomalies admitted to a pediatric unit. This is a quantitative and retrospective research. The sample consisted of records of children aged from 0 to 12 years old who were hospitalized in the pediatric unit of the University Hospital of Londrina, Paraná, Southern Brazil, from 2007 to 2009. Data were collected by chart review processed in the Epi-Info® program, and they were submitted to descriptive statistical analysis. There were 282 first admissions of children with congenital anomalies. Of these, 130 (46.0%) required readmission totaling therefore 412 hospitalizations. The mean stay was three days. There was a male predominance, and the age range was from three to seven years old. The most frequent congenital anomalies were in the genital and urinary systems (19.6%), followed by cleft lip and palate (17.3%), and those of the circulatory system (16.2%). There were 269 surgical procedures to correct there anomalies. This ...